Growth factors in cystic fibrosis

Introduction: Cystic fibrosis is one of the most common autosomal recessive diseases that affects sweat glands and mucosa. CF is a hereditary disease with annual incidence of about 2500 new cases in United Kingdom. Insulin-like growth factor-1 [IGF-1] and insulin-like growth factor binding protein-3 levels decrease in CF. The aim of this study was to assess the role of growth peptides in patients with CF

Method: We searched PubMed, Google scholar, IranMedex, and Scientific Information Database [SID] in September 2012 to April 2014. We included clinical studies with available abstracts and full texts that were in English or Persian languages. Manual searching was conducted within the reference lists of articles. Two reviewers independently applied eligibility criteria, assessed quality, and extracted data

Result: The earliest study was published in 1997 and the most recent one was in 2014. Study participants were adults in 3 studies [20%] and 12 studies [80%] were conducted in children. Patients with CF have lower levels of IGF-1 and there is a significant correlation between IGF-1 levels and growth index in patients with CF

Conclusions: IGF-1 decreases in children with CF and might be the cause of poor growth and low body mass index in these children

Effects of iron supplementation twice a week on attention score and haematologic measures in female high school students

<p><b>INTRODUCTION</b>Iron deficiency, associated with a decline in cognitive function, is the most common nutritional deficiency globally. The present study aimed to identify the impact of weekly iron supplements on the attention function of female students from a high school in North Khorasan Province, Iran.</p><p><b>METHODS</b>This was a blind, controlled, clinical trial study, involving 200 female students who were chosen using the stratified randomised sampling method. First, laboratory studies were performed to detect iron consumption limitations. Next, the 200 students were divided randomly and equally into case and control groups. The case group was treated with 50 mg of ferrous sulfate twice a week for 16 weeks. We compared both groups' data on attention, iron status and erythrocyte indices. Questionnaires were used to collect demographic data, while clinical data was collected using complete blood count and Toulouse-Piéron tests. Data was analysed using descriptive statistics, as well as paired and independent t-tests.</p><p><b>RESULTS</b>The mean attention scores of the case and control groups were 104.8 ± 7.0 and 52.7 ± 9.6, respectively (p < 0.001). The mean haemoglobin levels of the two groups were 12.5 ± 0.9 and 11.2 ± 1.0, respectively (p < 0.001). Compared to the control group, the attention scores and haemoglobin concentrations of the case group were found to be improved by approximately 90% and 10%, respectively.</p><p><b>CONCLUSION</b>Oral iron supplements (50 mg twice a week for 16 weeks) were able to improve the attention span and haematologic indices of female high school students.</p>

Synbiotics in children with cow's milk allergy: a randomized controlled trial

Cow's milk protein allergy usually occurs in infants within the first months of life. It can affect several organs, but gastrointestinal symptoms are the most clinical symptoms observed. The most effective treatment is restricting the cow 's milk protein in mother and infant's diet. Lactobacillus GG supplementation in infant could be effective through modulation of the immune system and the gut microflora. Thirty two breastfed infants with Cow's milk protein allergy were enrolled in a double-blinded randomized controlled trial in which they received Synbiotic [n=16] or placebo [n=16] once a day for one month, simultaneously with Cow's milk protein restriction in mother and infant's diet. Clinical gastrointestinal symptoms [vomiting, colic, rectal bleeding and diarrhea], head circumference, body length and weight were recorded at the beginning, the end of the first and third month of study. Percentage of increment in head circumference and weight were statistically more in synbiotic group compared with placebo group at the end of the first and third month of study. There was no significant difference in resolution of clinical gastrointestinal symptoms [vomiting, colic, rectal bleeding or diarrhea] and percentage of increment in body length. Synbiotic supplementation in infants may improve increment of head circumference and weight gain, but has no effect on resolution of clinical symptoms

Synbiotic for prevention of antibiotic-associated diarrhea in children: a randomized clinical trial

Antibiotic- associated diarrhea is a common problem in pediatric population. There is growing interest in probiotics, probiotics and synbiotics for prevention of this complication because of their worldwide availability as dietary supplements. The aim of this study was to assess the efficacy of a synbiotic mixture in prevention of antibiotic- associated diarrhea. In this randomized controlled trial, 218 patients [111 in the synbiotic and 107 in the placebo group] aged 6 months to 14 years with respiratory tract infection and/ or otitis media who needed antibiotic treatment in outpatient setting, were enrolled. They received 1 billion Colony Forming Unit of seven probiotics species plus Fructooligosaccharide in form of powder or placebo [matched for size, shape, and volume] for 7 days. Amoxicillin, Amoxicillin-clavalanic acid, cefixim and Azithromicin were the most common drugs used by physcicians Mothers recorded stool frequency and consistency daily for 7 days. We found no significant difference [P>0.05] in occurrence of diarrhea between synbiotic and placebo groups. This synbiotic mixture did not appear to reduce antibiotic- associated diarrhea in children. Further studies are needed to investigate the potential benefits of Synbiotics in prevention of this disease

Utility of modeling end-stage liver disease in children with chronic liver disease

Chronic liver diseases consist of wide spectrum disorders that may be complicated by cirrhosis and therefore need to transplantation. The pediatric end-stage liver disease [PELD] score and model of endstage liver disease [MELD] score has been used as predictors of mortality chronic liver diseases listed for liver transplantation. The aim of this study is evaluation of relation between PELD MELD score and evidence of cirrhosis in children with choronic liver disease. This cross-sectional study conducted on 106 patients of chronic liver disease referred to Ghaem haspital, Mashhad university of medical science, Iran during 24 months period [2010-2013]. PELD and MELD score were calculated for all patients. Clincal and patholoogical findings of cirrhosis were recorded. Mean age of patients was 68/3 +/- 41.8 months. Mean PELD MELD score was -1/59 +/- 9/64. There was significant correlation between PELD MELD score and clinical icter, spelenomegaly, evidence of hepatopulminary syndrome, esophageal varices, evidence of cirrhosis in tissue specimences. PELD MELD score appear to be benefit for detection of cirrhotic children among paients with choronic liver disease

Characteristics and outcome of three forms of autoimmune hepatitis in Iranian children

To define the clinical features, biochemical and histological findings and outcome of three forms of autoimmune hepatitis. In a cross sectional study between November 2001 to January 2008 in Tehran and Mashhad university of medical sciences, 61 children who diagnosed as AIH [40 girls and 21 boys] have been analyzed for their clinical, serological, and histological data. Variables analyzed included age, sex, clinical presentation, hepatic function, immunoglobulins, autoimmunity markers, histology and clinical evolution. According to the auto-antibodies profile, AIH patients were classified as type I AIH [ANA or smooth-muscle antibodies or both positive], type II [anti-LKM-1 positive] and type III [antibody negative]. We analyzed data of 61 children with AIH. 51 patients [median age: 10 [0.7-14] years had anti-nuclear and/or smooth muscle antibody [ANA/ASMA or both], 5 [median age: 7 [2-8] years] had liver/kidney microsomal antibody [LKM-1]. 5 patients had no detectable autoantibody. At presentation: 60% had jaundice or symptoms of acute hepatitis; LKM-1 positive were younger. Interface hepatitis was seen in 100% and fibrosis +/- cirrhosis were found in 70%. Frequency of AIH was 2:1 in girls. Type 1 was the most frequent diagnosis [83%] and was more frequent in older children. Relapse and treatment failure were common in type II. AIH may have different clinical presentation in children. Althout most of cases may have high inflammatory activity or cirrhosis, good response to treatment and remission may accur

Determine efficacy of a short course of montelukast in children with intermittent asthma and viral infection

Mild intermittent asthma is common in children and viral infections are responsible for the majority of exacerbations. As leukotrienes are potent inflammatory mediators, some studies have shown that Montelukast, a leukotriene receptor antagonist, may be effective on reduction of asthma symptom. To determine whether a short course of Montelukast in asthmatic children with common cold would modify the severity of an asthma episode. Children, aged 6-12 years with intermittent asthma participated in this randomized, double-blind, placebo-controlled clinical trial. Treatment with Montelukast or placebo was initiated at the onset of viral upper respiratory tract infection and continued for 7 days. Primary outcomes included the clinical manifestation: duration of episodes, daily symptom, nights symptoms and activity limitation. Secondary outcomes included the need for beta agonist usage, oral prednisolone, physician visit, hospital admission and school absence. A total of 187 children with intermittent asthma were randomized, 93 to Montelukast group and 94 to placebo group. Montelukast significantly decreased the cough by 17.3% [P<0.001], nighttime awakenings by 5.4% [P=0.01], interference with normal activity by 6% [P<0.01], time off from school by 6% [P<0.01], beta-agonist usage by 17.2% [P<0.001] and doctor visits by12.2% [P<0.01] compared to placebo. Whereas there was a non significant reduction in wheezing, tachypnea, respiratory distress, asthma exacerbation, oral prednisolone and hospitalization [P=0.8]. A short course of Montelukast, introduced at the first sign of a viral infection, results in a reduction in cough, beta-agonist use and nights awakened, time off from school and limitation of activity. More studies are needed to evaluate the optimal dose and duration of treatment

Kawasaki disease presenting as acute clinical hepatitis

Kawasaki disease is a systemic vasculitis of children. Among gastrointestinal symptoms of this disease jaundice occurs uncommonly. We present a 23 month boy with icter and clinical hepatitis and final diagnosis of kawasaki disease

Ultrasonographic triangular cord sign and gallbladder abnormality in diagnosis of biliary atresia

Early diagnosis of biliary atresia is very important for better outcome of treatment. Ultrasonography is one of the diagnostic tools for early differentiation of biliary atresia from other causes of neonatal cholestasis. It has been reported that triangular cord sign [TACS] in sonography is a reliable sign for diagnosis of biliary atresia. The aim of this study was to re-assess the accuracy of TACS alone and coupled with an abnormal gallbladder in the diagnosis of biliary atresia. Infants with prolonged cholestatic jaundice underwent ultrasonography and liver biopsy. Results of ultrasound scans [TASC and gallbladder abnormality] were compared with histopathological findings. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of Ultrasonographic findings for diagnosis of biliary atresia based on liver histopathology were studied. The sensitivity, specificity and accuracy of TACS for diagnosis of biliary atresia were 36%, 95% and 77% respectively. Positive predictive value and negative predictive value was 77%. The sensitivity and specificity of gallbladder abnormality for diagnosis of biliary atresia were 36% and 88% respectively. Positive predictive value of TACS coupled with gallbladder abnormality for diagnosis of biliary atresia was 100%. The Ultrasonographic TACS is very specific for diagnosis of biliary atresia. Positive predictive value and accuracy of this sign is much higher than of gallbladder abnormality. The sensitivity of TACS for diagnosis of biliary atresia is very low

Prevalence of portal vein thrombosis following umbilical catheterization in neonatal period

Portal venous thrombosis [PVT] is one of the most common causes of extrahepatic portal hypertension in children that may be occurring following umbilical venous cauterization or omphalities during neonatal period. We investigated the effects of umbilical cauterization during neonatal period on portal vein thrombosis. This study investigated the frequency of thrombosis of portal vein in term or preterm infants following umbilical catheterization by color doppler ultrasound. Fifty neonates who had age range [3.6+ 8days] and birth weights 1250- 4230 gram were recruited for this study and umbilical venous catheters were placed on neonates. All parents of these neonates signed a consent form. Color doppler ultrasound sonography of the portal vein was performed by two expert radiologists within 3 days following umbilical cauterization and serially for at least a period of 6 months intervals up to 3 years [6 times for each case]. Ten children were excluded from the study because of lack of follow up. Forty children completed the study. From total of 40 subjects, portal vein thromboses were detected in two children [1 girl, 1 boy] with age 2.5 and 1.5 years respectively. In these two children, other clinical signs of portal vein thrombosis manifested such as esophageal and stomach varices and splenomegaly confirmed by endoscopy. In all the other children, the physical examinations and ultrasounds were normal during 6 stages. It is recommended that care be exercised during cauterization placement in order to prevent thrombosis of the portal vein from occurring

Role of pulse oximetry in detecting mild to moderate hepatopulmonary syndrome in children

Hepatopulmonary syndrome [HPS] refers to arterial hypoxemia caused by pulmonary vasodilation, which is a consequence of portal hypertension. HPS is associated with increased morbidity and mortality; thus, it is important to diagnose this entity as soon as possible for treatment to be administered. In a cross-sectional study, 40 children [6 months to 14 years old] with chronic liver disease were enrolled. In all patients, measurements of Oxygen saturation [SaO[2]] were performed with a pulse oximeter in the supine position [SPO[2]] and then in the upright position [delta SPO[2]]. Children were divided into three groups: i] those with both SPO[2]>96% and deltaSPO[2]>4%; ii] children with either SPO[2]>96% or delta SPO[2] > 4%; and iii] those with neither of these signs. Then, contrast-enhanced echocardiography [CEE] and arterial blood gas [ABG] were performed. Finally, the prevalence of mild to moderate HPS was calculated in the three groups. There were 30 patients who had neither of the two signs, of which 9 had HPS. Ten patients had one of the two signs, in whom 4 had HPS. None of the patients had both signs. The sensitivity of the pulse oximetry was 30%, specificity was 77%, positive predictive value was 38% and negative predictive value was 70%.There is a significant prevalence of HPS in cirrhotic patients which effects prognosis. Based on our study results, we have determined that pulse oximetry could not be a reliable screening procedure in mild to moderate HPS. It is recommended to use gold standard tests [echocardiography and arterial blood gasometry] for the screening and diagnosis of HPS in children.

Comparison between batch and fed-batch production of rhamnolipid by Pseudomonas aeruginosa

This paper presents a comparison between batch and three different sets of fed batch fermentations for rhamnolipid production by Pseudomonas aeruginosa. The batch run was performed with 500 ml of culture medium having the initial glycerol and sodium nitrate concentrations of 30 and 8.3 g/l, respectively. For a fed batch run with nitrogen source in feed, 250 ml of the nitrogen excluded culture medium was in the bioreactor initially, and 250 ml culture medium containing 16.6 g/l sodium nitrate was fed to the bioreactor continuously. A similar procedure was repeated for fed batch runs with carbon, and phosphorus source in feed. Statistical analysis showed that fed batch runs were better than batch in term of rhamnolipid production, and among the fed batch runs the maximum amount of rhamnolipid. [4.12 g Rhamnose Equivalent/l] was for the fed batch run with the carbon source in feed

[Endocrine disorder in children with chronic liver diseases]

An important consequence of chronic liver disease [CLD] is endocrine disorders. The main object of this study was to assess serum hormones in children with CLD. In a cross sectional study between 2007-2009, 57 children with chronic liver disease were enrolled. Levels of serum triiodothyronine [T3], thyroxin [T4], thyroid stimulating hormone [TSH], parathormon [PTH], calcium[c], phosphor [P], alkalanephosphatase [ALP],growth hormone[GH] and insulin like growth factor-1[IGF-1], albumin [ALB] and total protein, triglyceride [TG] and cholesterol [CHOL], fasting blood sugar [FBS] and liver function tests were measured. In this study, most patients' thyroid hormones and fasting blood sugar were at normal range. But there was a significant decline in serum IGF- 1 levels in this patients. 28 percent of children had PTH levels higher than normal range for age and 24 percent had serum phosphor levels lower than normal range .1/3 of these patients were bone fracture. Overt diabetics did not obsereve in our children. Children with CLD are GH resistant and might be at risk of osteopenia and bone fracture. So serum hormone levels should be carefully monitored in children with CLD

Predictive factors of pediatric intractable seizures

This study was performed in children aged <15 years, at the Pediatric Neurology Clinic of Imam Reza Hospital affiliated to Mashhad University of Medical Sciences. The objective of this study was to recognize the main predisposing factors that result in uncontrolled seizures in patients so that we can start the treatment accurately. There were two groups of patients; group I, consisted of 51 patients, with minimum refractory seizures of one episode per month while taking at least two antieplieptic drugs, and group II, comprised of 80 well-controlled patients chosen at random, who had no fit within 6 months after starting the treatment. Factors affecting the occurrence of refractory seizures included age <1 year, multiple seizures before starting the treatment, male gender, myoclonic seizures, neurologic defects, neonatal and daily seizures, and first abnormal electroencephalogram and brain computerized tomo-graphy scan. There are several factors that can predict development of uncontrolled seizures. Knowledge of these factors helps us to discriminate our patients and pay more attention to those at risk of developing uncontrolled seizures